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Biomedical subjects

D D Sherry

Publications and source records attributed to D D Sherry.

At least 37 records · Page 2Linked to original sources

Reiter's syndrome associated with cryptosporidial gastroenteritis.

We describe a child with Reiter's syndrome (RS) associated with cryptosporidial gastroenteritis. Two children have recently been reported with reactive arthritis due to Cryptosporidium: however, our child represents the first pediatric case of RS secondary to cryptosporidiosis.

Arthritis, Reactive↗

A broadened spectrum of juvenile myositis. Myositis-specific autoantibodies in children.

OBJECTIVE: Myositis-specific autoantibodies (MSA) define relatively homogeneous clinical and immunogenetic patient groups in adults with idiopathic inflammatory myopathies (IIM). This study explores the usefulness of MSA in defining groups of children with myositis. METHODS: Sera from 77 children with myositis and other connective tissue diseases were tested for MSA by immunoprecipitation and immunodiffusion. Clinical data were collected and analyzed. RESULTS: The MSA anti-PL-12 (alanyl-transfer RNA synthetase), anti-Jo-1 (histidyl-tRNA synthetase), anti-signal recognition particle, and anti-Mi-2 were each identified in the sera of 12 children with IIM. In these patients, the clinical manifestations, disease courses, and responses to therapy closely resembled those in adults with the same autoantibodies. CONCLUSION: These observations suggest that the clinical syndromes defined by particular MSA are similar in children and adults with IIM. By defining similar clinical syndromes in children who have MSA, this study provides a basis for future studies of MSA in the idiopathic inflammatory myopathies of childhood, which may be useful in predicting the clinical courses of a subset of these patients and improving their therapy.

Adolescent↗

Stroke and mixed connective tissue disease.

We describe the clinical presentation and course of two girls with cerebrovascular disease and mixed connective tissue disease. One developed rapid onset hemiparesis and aphasia secondary to left internal carotid artery occlusion. She experienced a complete recovery after treatment with prednisone and cyclophosphamide. The other patient was diagnosed as having mixed connective tissue disease but had acute neurologic deterioration. She died due to an intracerebral hemorrhage. Autopsy demonstrated small-vessel fibrinoid necrosis. Although cerebrovascular disease secondary to central nervous system vasculitis is a manifestation of systemic lupus erythematosus, this is the first description of cerebrovascular disease as a primary sign in mixed connective tissue disease. These cases demonstrate the range of cerebrovascular disease observed in children with mixed connective tissue disease.

Adolescent↗

Intraarticular alpha 2-macroglobulin complexes and proteolytic activity in children with juvenile rheumatoid arthritis.

In juvenile rheumatoid arthritis (JRA), it is likely that the release of proteolytic enzymes from activated synovial fluid neutrophils overwhelms the major protease inhibitor, alpha 2-macroglobulin (alpha 2-MG), and leads to cartilage destruction. Due to the unique nature of the alpha 2-MG-protease complex, proteolytic function is maintained until the complex is cleared. In this study, we sought to determine the concentration of alpha 2-MG-protease complexes in synovial fluid of patients with JRA, the proteolytic activity found in their synovial fluid, and whether the alpha 2-MG complexes are associated with increased proteolytic activity. The JRA patients' synovial fluids had complex levels of 217.0 +/- 192.2 nmol/L--significantly elevated compared with plasma values (p < 0.001) and with control synovial fluid (p < 0.05). Elastase activity (almost entirely neutrophil elastase) was detected in all JRA synovial fluid samples (mean 2.9 +/- 2.6 mg/L) and significantly correlated with alpha 2-MG-complex values (r = 0.67, p < 0.01). Synovial fluid tryptic activity was detectable in all JRA patients but did not significantly correlate with alpha 2-MG complexes (r = 0.53, p > 0.05). Seventy-four percent of total elastase activity and 41% of total tryptic activity were contained in the alpha 2-MG-complex fractions. We suggest that the increased concentration of synovial fluid alpha 2-MG complexes with retained elastase activity contributes to continued proteolysis and joint destruction and may affect the subsequent disease course through its role as a modulator of IL-6.

Adolescent↗

Pilot investigation of naproxen/methotrexate interaction in patients with juvenile rheumatoid arthritis.

OBJECTIVE: To investigate the potential interaction of naproxen and methotrexate (MTX) in children with juvenile rheumatoid arthritis (JRA). METHODS: Nine children with JRA served as their own control taking their usual doses of MTX (0.22-1.02 mg/kg/week) and naproxen (14.6-18.8 mg/kg/day) separately and in combination. RESULTS: MTX affected a > or = 30% change in naproxen kinetics in 6/8 patients, while naproxen altered MTX kinetics by > or = 30% in 4/9 patients. CONCLUSION: MTX can alter nonsteroidal antiinflammatory drug (NSAID) kinetics in children with JRA and NSAID can alter MTX kinetics. NSAID toxicity should be considered when assessing adverse reactions in patients receiving the combination treatment of MTX and NSAID:

Adolescent↗

Treatment of neonatal lupus: case report and review of the literature.

A critically ill infant with congenital complete heart block and neonatal lupus was treated with pulse steroids, exchange transfusion and intravenous gammaglobulin. Transient improvement in clinical status and laboratory results occurred, although the infant died. Based on this report and review of prior experience with immunosuppressive therapy, prenatal treatment of the mother with betamethasone or dexamethasone has been successful in resolving the cardiomyopathy/myocarditis associated with neonatal lupus. Postnatal treatment of the infant with steroids appears to be beneficial for persistent hepatic and hematologic manifestations. The evidence for exchange transfusion is less certain.

Betamethasone↗

Predicting remission in juvenile rheumatoid arthritis with methotrexate treatment.

Forty-nine children with a polyarticular course of juvenile rheumatoid arthritis treated with methotrexate (MTX) for at least one year were analyzed to identify clinical characteristics that would predict remission of arthritis after MTX treatment. Twenty-two children (45%) had remission of arthritis after a mean of 13.6 months of treatment and did not differ from the 27 with persistently active arthritis regarding years of disease before starting MTX, age starting MTX, maximum MTX dose, disease onset type, presence of radiographic joint destruction, concomitant treatment with hydroxychloroquine, sulfasalazine or prednisone, or presence of rheumatoid factor or antinuclear antibodies. Higher dose MTX, earlier treatment, genetic markers, and a standardized route of therapy may yield important information in future studies.

Arthritis, Juvenile↗

Preliminary report of higher dose methotrexate treatment in juvenile rheumatoid arthritis.

Methotrexate (MTX) is widely used to treat juvenile rheumatoid arthritis (JRA). Although most patients respond to lower doses (0.15-0.5 mg/kg/wk), some patients have required higher doses of MTX to control their arthritis. Thirteen children were treated with MTX 0.82-1.1 mg/kg/wk for 2-26 months. Although all children initially responded (> 50% improvement in joint index, erythrocyte sedimentation rate, morning stiffness and global evaluation), 4 patients discontinued treatment because of side effects or lack of prolonged efficacy. Five patients have continued taking higher dose MTX for 4-26 months, while 4 other patients have been able to decrease their MTX dose and maintain improvement. Twenty-four hour MTX levels were done on all patients at initiation of higher dose MTX and all cleared MTX well. Our report suggests that MTX in doses of 0.82-1.1 mg/kg/wk can successfully treat active synovitis in some children with severe JRA with few short term toxicities. Longterm use at these doses has not been studied and thus its safety is not known.

Adolescent↗

Decreasing severity of chronic uveitis in children with pauciarticular arthritis.

We compared the current prevalence and severity of chronic uveitis in children with pauciarticular juvenile rheumatoid arthritis in Seattle, Wash, with that of children with the same condition in the same area in 1975. The prevalence of eye disease decreased from 45% in 1975 to 13% in 1989, and the proportion of patients with severe visual loss decreased from 21% in 1975 to none in 1989. We could not attribute these findings to differences in known risk factors for iritis, such as age, sex, or presence of antinuclear antibodies. There was no difference in the duration of follow-up between the two groups. It is possible that the decline in prevalence of uveitis reflects a referral bias for eye disease in the 1975 population. However, the decrease in disease severity remains unexplained and may represent more effective treatment, earlier surveillance for ocular disease, or a change in the frequency of ocular manifestations of this disease in the 1989 group.

Arthritis, Juvenile↗

Psychosomatic musculoskeletal pain in childhood: clinical and psychological analyses of 100 children.

The clinical and psychological findings on 100 children with psychosomatic musculoskeletal pain seen at a major pediatric rheumatology referral center are reported. Most (76%) were female, median age was 13 years, and median duration of symptoms was 1 year. Multiple painful sites were common (66%). The pain was constant (63%) or intermittent (37%); 45% had hyperesthesia, and almost all maintained a cheerful affect when complaining of severe pain. Two predominant abnormal family milieu were seen. One was cohesive, stable, and organized, but intolerant of separation and individuation. The other was chaotic, emotionally unsupportive, with high levels of conflict. Members of the cohesive family type reported significantly less distress than members of chaotic families. Enmeshment between mother and child was common in both family types. Although frequently viewed as bright, most of these children had normal intelligence, and some had unrecognized academic difficulty. These children, compared with those with arthritis, had a significantly lower global well-being score. Clinical depression was unusual (11%). Most (97%) responded favorably to intensive physical and occupational therapy along with individual or family psychotherapy; 78% become symptom free or fully functional. Children with these signs and symptoms should have full psychological evaluations and respond well to treatment directed toward decreasing pain and restoring function.

Adolescent↗

Shoulder limitation in juvenile rheumatoid arthritis.

One hundred consecutive children with juvenile rheumatoid arthritis (JRA) were evaluated for shoulder dysfunction. Shoulder arthritis was virtually absent in all 45 children with pauciarticular onset JRA. Twenty of 40 children (50%) with polyarticular JRA and 12 of 15 (80%) with systemic onset JRA had shoulder involvement characterized by pain or restricted passive range of motion (PROM). Ninety-five percent of those with shoulder arthritis had bilateral involvement. Children with systemic onset were likely to have shoulder disease within 2.5 years of onset and to have more severely limited PROM. Children with polyarticular onset developed shoulder arthritis any time during the course of their disease. With either type of onset, internal rotation was the most commonly and severely limited motion, followed by abduction. Clinicians treating children with JRA should carefully monitor pain and examine both rotational and planar components of shoulder motion.

Adolescent↗